Perianal Paget's disease is a rare malignant condition affecting the epidermal apocrine gland that is usually complicated, with a confusing clinical picture, and diagnosis is therefore often delayed. Although, more than 100 years has gone by since its first description by Darier and Coulillaud in 1893, proper treatment guidelines have yet to be established.OBJECTIVE
To review the available literature, emphasizing various current treatment strategies and aiming to increase clinicians' awareness of this rare disease, along with its management, for better practice and improvement of patient prognosis.METHODS
The review of the concerned literature was done by searching and compiling data available on PubMed, Medline, and other databases using the key words “perianal Paget's disease,” “extramammary Paget's disease,” “intraepithelial adenocarcinoma,” and “apocrine glands.”CONCLUSIONS
Surgery is the mainstay of treatment. Some noninvasive therapeutic modalities were also reported to be effective, such as photodynamic therapy, imiquimod, radiotherapy, chemotherapy, anti-androgen therapy, and carbon dioxide. Management of perianal Paget's disease remains difficult, and large controlled, multicentric studies should be performed to compare the effectiveness of current thera-peutic modalities.